Successful Treatment of a Patient with Lhermitte-Duclos Disease (a Case Report and Literature Review)

Lhermitte-Duclos disease, also known as dysplastic cerebellar gangliocytoma, was first diagnosed by doctors Lhermitte and Duclos in 1920. In subsequent years, the disease was described by many researchers: Bielschowsky and Simons in 1930, Christensen in 1937, Duncan and Snodgrass in 1943, Ambler in 1969, and Padber in 1991. Each of the authors referred to this disease in a different way: diffuse gangliocytoma of the cerebellum, purkinjeoma, hamartoma, dysplastic gangliocytoma, diffuse ganglioneuroma, and granule cell hypertrophy of the cerebellum. In total, about 220 cases of Lhermitte-Duclos disease have been described in the literature [1]. In 1920, Lhermitte and Duclos described a disease in a 36-year-old male who first developed symptoms of hearing loss on the left and occipital pains at the age of 10 months. Before admission, the patient had paroxysmal vertigo with repeated falls. Examination revealed cerebellar ataxia, dysarthria, nystagmus, and neuropsychic disorders with disorientation and memory impairment. One week after admission, the patient's condition deteriorated. He was not operated on; the patient’s consciousness level gradually decreased to a coma, and he died. Considering the disease nature, Lhermitte and Duclos suggested that a pathological lesion found in the posterior cranial fossa during autopsy was a combination of a congenital malformation and a tumor developed from ganglion cells [1, 2]. In 1937, Christensen reported the first successful surgery for dysplastic gangliocytoma of the right cerebellar hemisphere. The surgery was carried out in a 34-year-old male who had suffered the disease since the age of 6 years. The patient had rises in blood pressure, occipital and temporal headaches, nausea, and vomiting at the peak of headache. Sometimes these attacks resulted in the loss of consciousness. The findings at admission included bilateral papilledema, trigeminal nerve impairment on the left, and the right facial paralysis. Ventricu-